Life with CGD

Over the last 60 years, Chronic Granulomatous Disease (CGD) has taught many lessons in basic science, infection susceptibility, and the role of inflammation in immune responses. We have come a long way in these 60 years, to an era of greatly improved survival into adulthood and extraordinary improvements in quality of life, where there is potential for cure by bone marrow transplantation, and where there are early phase experimental clinical trials of gene therapy. In the 1950’s CGD was characterized as a fatal disorder of young children involving susceptibility to severe infections with formation of granulomas (collections of inflammation related blood cells). Improvements in diagnosis of infection, and the availability of more potent antibiotics in the 1960’s resulted in significantly improved survival of patients with CGD. Also in the 1960’s it was first appreciated that CGD was a defect in the ability of certain white blood cells, called neutrophils, to produce hydrogen peroxide and other substances important to control bacterial and fungal infections.

With use of prophylactic antibiotics and other advances in the 1970’s and 1980’s, most CGD patients had long periods of time infection-free. It was also appreciated by that time that there are several genetic sub-types of CGD, that girls as well as boys could be affected by some forms of CGD, and that there were both mild and severe forms of CGD. By the 1990’s the currently accepted regimen of preventative prophylaxis for infection had been established, and with enhanced methods to diagnose and treat infection, the great majority of children with CGD could expect to attend college, reach adulthood, hold jobs, get married and have children.

In the current era the outlook for CGD is very encouraging, in that the majority of patients with CGD, even those with the most severe forms of CGD, may have two or three years between severe infections, and those with milder forms of CGD may even go a decade or more between severe infections. With prolonged infection-free survival, it is now the non-life threatening chronic inflammation problems such as inflammatory bowel disease that for some patients is more concerning to them than the risk of infections. Many people with CGD can carry on a normal life with few problems.1

However, patients with CGD remain at significant risk for infection throughout life. They must take their prophylactic medications to reduce risk of infection, remain cautious with respect to situations that could increase risk of infection, and get early diagnosis and treatment for possible infections.

Hospitalizations may be required for patients with CGD to locate sites and causes of infections. Intravenous antibiotics may be needed for serious infections. Prophylactic antibiotics and treatment with interferon gamma increase healthy periods. The vast majority of patients reach adulthood, when serious infections tend to occur less frequently.

Individuals with X-linked CGD are known to be prone to having short stature, and the reason for this is unknown. That is not true for the other molecular types of CGD.

Avoiding Infection

While many patients with CGD are leading full and active lives, it is important to avoid certain activities that can expose patients to heightened risks of infection.

The following are several precautions to keep in mind:
  • Many physicians suggest that swimming should be confined to well-chlorinated pools. Ponds and brackish water in particular may expose patients to organisms that are specifically dangerous in CGD. Swimming in even large lakes and rivers carry some risk. Ocean bathing in clean areas appears to have lower risk than swimming in bodies of fresh water and rivers.
  • Always wear shoes outdoors.
  • A major risk is the handling of garden mulch (shredded moldy tree bark) or potting soil. This type of exposure can cause a severe life-threatening pneumonia due to inhalation of the fungus Aspergillus, or to nocardia and some other bacteria, which like to live in decaying plant matter or soil.
  • Patients with CGD should remain indoors during mulching in neighboring yards. Once the mulch is settled firmly on the ground and is not being spread or raked, it is much less of a danger to patients with CGD.
  • Patients should avoid turning manure or compost piles, repotting house plants, cleaning cellars or garages, removing carpets, performing demolition, digging in dirt, dusty conditions, cutting grass, raking leaves, hay rides and barns.
  • Look for playgrounds without woodchips, as they can harbor fungi. Asphalt, rubber mats, and grass surfaces are fine. The risk here is more problematic for small children who may dig in or play with the woodchips. The risk to teenagers or adults from woodchip playgrounds is quite minimal from simply walking or running over the woodchips, and is no more risky than a walk on a path in the woods.
  • In general, patients with CGD should avoid sports or activities that involve kicking up or playing in dust and dirt. Baseball, for example, is not recommended for patients with CGD, as “sliding into base” involves direct contact with dirt and dust. Patients with CGD report that they enjoy participating in a wide array of sports and outdoor activities, such as biking, yoga, tennis, running/jogging, ice hockey, riding scooters, skateboards, and golf. Even soccer played on a grassy field is likely a safe sport for CGD patients.
  • Aspergillus is present in many samples of marijuana, so patients with CGD should avoid it.
  • While tobacco smoking is a health risk to everyone, patients with CGD already have a tendency to develop excessive inflammation, and smoking may exacerbate this tendency for increased inflammation and damage to lungs in CGD.

For more information about programs and resources for individuals and families living with CGD, contact IDF via Ask IDF or 800-296-4433.

  • IDF Friends, is our social network created exclusively for adult patients and family members. You can connect with others in the CGD group. Create an account and start connect with others. Click here to visit IDF Friends.
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